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Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematological cancer usually involving skin and/or bone marrow. Recent molecular evidence suggests that BPDCN originates from the myeloid lineage. Shortterm responses to conventional treatments underline the need for new therapies in this disease. We present the case of a 55-year-old woman with therapy-related leukemic-phase BPDCN who experienced a six month remission following clofarabine and cytarabine combination therapy. Intensive anthracyclin-based regimen and stem-cell transplant (SCT) were excluded due to pre-existing severe cardiac dysfunction. Relapse occurred after six month of complete remission and the patient died from thrombocytopenia-induced intracranial bleeding. For elderly or young BPDCN patients not eligible for intensive regimen or in relapsed/refractory disease, clofarabine-based therapy appears as an option.

Frontline Clofarabine and Cytarabine combination for plasmocytoid blastic dendritic cell neoplasm